Bile Duct Cancer (Cholangiocarcinoma)

Cholangiocarcinoma is a type of cancer located in the liver’s bile ducts, which carry bile to the small intestine. Although it grows slowly, it often remains undetected until its advanced stages, when it blocks the bile duct and produces symptoms.

Key Facts

  • Cholangiocarcinoma, also known as bile duct cancer, is a rare disease that usually only affects those aged 50 or older.
  • It grows in the liver’s bile ducts, which carry bile (a digestive fluid) to the gallbladder and small intestine. 
  • If it is discovered early enough, surgery may be able to remove the cancer completely. If not, radiation therapy, chemotherapy, and other treatments are available.


Like other cancers, bile duct cancer occurs when cells develop DNA mutations that cause them to multiply uncontrollably. This eventually forms a mass of cancerous cells called a tumor. While it’s not clear what causes cells to develop these mutations, there are risk factors that may make it more likely. These include:

  • Bile Duct Cysts: These can block the flow of bile, causing inflammation and infection, which increases the likelihood of cancer.
  • Liver Inflammation: This can be caused by hepatitis C, NASH, and other diseases.
  • Older Age: People 50 years and older are more likely to develop bile duct cancer.
  • Primary Sclerosing Cholangitis: This condition causes inflammation and scarring in the bile ducts, which increases the risk of cancer.
  • Parasitic Infection: In parts of Asia, parasites called liver flukes can increase the risk of developing bile duct cancer.
  • Smoking: Smoking increases the risk of cancer throughout the body.
  • Ulcerative Colitis: This condition can also cause inflammation of the bile ducts, which increases the risk of cancer.

Having one or more of these risk factors does not necessarily mean cancer will develop. In fact, bile duct cancer is extremely rare, occurring in only 2 out of every 100,000 people. 


The following are some of the most common symptoms of bile duct cancer:

  • Abdominal pain
  • Fever
  • Itchy skin
  • Jaundice (yellowing of skin and eyes)
  • Light-colored stools
  • Nausea or vomiting
  • Weight loss


The first step to diagnosing bile duct cancer (or any cancer) is a physical examination. A doctor or medical professional will assess the patient’s general health, check for any signs of cancer, and review past treatments and medical history. The results of this exam will determine other diagnostic tests and treatments.

The following are some other common ways to diagnose bile duct cancer:

  • Blood Tests: These will measure levels of bilirubin, albumin, and other substances to determine how well the liver is functioning.
  • ERCP Test: This uses a thin tube with a tiny camera that is passed down the throat to the small intestine, allowing doctors to examine the bile ducts.
  • Imaging Tests: These will allow doctors to form an image of the liver so that they can better detect signs of cancer. They may include a computerized tomography (CT) scan, an ultrasound, and magnetic resonance imaging (MRI). 
  • Liver Biopsy: This is a surgical procedure that removes a tiny portion of liver tissue so that doctors can examine it under a microscope to determine what is wrong. A biopsy is the only test that can make a definite diagnosis of cancer.
  • Tumor Marker Test: This checks for a protein called CA 19-9 that bile duct cancer cells produce. High levels of this protein may mean cancer is present.


There are multiple ways to treat bile duct cancer. Which treatment or combination of treatments is best depends on the stage of cancer, the patient’s overall health, and possible side effects. 

Radiation Therapy 

This type of treatment uses high-energy radiation, such as photons or x-rays, to damage and destroy cancer cells. There are two forms of radiation therapy:

  • External Radiation Therapy: This directs a beam of radiation toward the tumor using a machine outside of the body.
  • Internal Radiation Therapy: This places a radioactive material inside the body using a needle or catheter, allowing doctors to target the cancer cells more precisely.


This type of treatment uses drugs to stop the growth of cancer cells and destroy them. It is also used to shrink the size of a tumor when surgery is not an option. It can be administered through the mouth or into the bloodstream. There are several different forms of chemotherapy treatment:

  • Systemic Chemotherapy: The drugs are injected into the bloodstream, allowing them to reach cancer cells throughout the body.
  • Regional Chemotherapy: The drugs are injected into an organ or a body cavity so that they will primarily affect cancer cells in a specific region.
  • Chemoembolization: The drugs are injected into small beads, which are placed inside an artery that feeds the tumor. This both blocks the tumor’s blood supply and increases the effectiveness of the drugs that reach it.

Chemotherapy drugs used to treat bile duct cancer include:

  • 5FU
  • Capecitabine
  • Cisplatin
  • Gemcitabine
  • Irinotecan
  • Oxaliplatin


Surgery involves removing the tumor and possibly some of the surrounding tissue. If the tumor is small enough, surgery may be able to remove it completely. In other cases, surgery may be used alongside other treatments, such as chemotherapy or radiation therapy.

Common types of surgery for bile duct cancer include:

  • Bile Duct Removal: This procedure is done if the tumor is small and has not spread beyond the bile duct. Lymph nodes may also be removed to check for signs of cancer.
  • Partial Hepatectomy: This procedure removes the section of the liver where the cancer is found. The amount removed will depend on the size of the tumor. Afterward, the liver will be able to regenerate within a few weeks or months.
  • Whipple Procedure: This is an extensive procedure that is done if the tumor is near the pancreas. It removes parts of the pancreas, stomach, small intestine, and bile duct. The remaining parts are then reconnected by the surgeon.
  • Liver Transplantation: This removes the entire liver and replaces it with a healthy donor liver. However, because bile duct cancer often recurs quickly after a liver transplantation, this procedure is rarely used. 


Successfully treating bile duct cancer depends on a variety of factors, such as where the tumor is located, whether it has spread to surrounding tissues or organs, and the patient’s health. If the cancer is located near the liver hilum (where the right and left bile ducts lead out of the liver) then treatment will be more difficult. Similarly, if the cancer has spread to the lymph nodes or beyond, it will be harder to remove. 

Survival Rates 

The American Cancer Society has calculated survival rates for bile duct cancer based on both location (i.e., inside or outside the liver) and severity (i.e., the spread of the cancer). Severity is divided into three categories: 

  • Localized: The cancer has not spread beyond the bile ducts.
  • Regional: The cancer has spread beyond the bile ducts to the lymph nodes.
  • Distant: The cancer has spread to other organs.

The five-year survival rate for bile duct cancer that begins inside the liver is:

  • Localized: 24 percent
  • Regional: 6 percent
  • Distant: 1 percent 

The five-year survival rate for bile duct cancer that begins outside the liver is: 

  • Localized: 13 percent
  • Regional: 17 percent
  • Distant: 1 percent 

Preventing Recurrence 

A medical and possibly surgical oncologist (doctors who specialize in treating cancer) will work with the patient to remove the cancer and prevent it from recurring. Since bile duct cancer can be aggressive and difficult to detect, this may involve both surgically removing cancerous cells and undergoing an extensive regimen of chemotherapy and/or radiation therapy. If cancer does return, a new round of testing will begin so that as much as possible is known about it. This will ensure that any additional treatment is as effective as possible.

Next Steps 

Our center specializes in diagnosing and treating all forms of bile duct cancer. We are one of the few centers in the country capable of performing surgeries and transplantations on tumors other hospitals deem ineligible for surgery. In addition, we also offer experimental procedures such as molecular and targeted therapy.

Call us at (877) LIVER MD/ (877) 548-3763 or use our online form to schedule an appointment.